The Authors present 9 patients affected by Dysplasia Epiphysealis Hemimelica with a mean follow-up of 11 years (range 5 years-21 years). The Authors confirm the benignity of the disease and report the variability of the clinical evolution of this rare type of osteochondrodysplasia. Besides cases with spontaneous evolution characterized by no clinical evidence of the disease, there are cases that require surgical removal of the osteochondromas and, sometimes, corrective interventions because of axial deviations and/or limb discrepancy.